Deconinck Wouter, Bamps Sven, Steelandt Thomas, Wissels Maarten, Plazier Mark, Put Eric, Achahbar Salah-Eddine, Vanvolsem Steven, Meeuws Sacha, Klein Sam, Roosen Gert
Department of Neurosurgery, Jessa Hospital, Hasselt, Belgium.
Department of Neurosciences, Faculty of Medicine, University of Hasselt, Hasselt, Belgium.
Surg Neurol Int. 2024 Nov 15;15:415. doi: 10.25259/SNI_640_2024. eCollection 2024.
Myeloid sarcoma (MS), a rare extramedullary tumor composed of myeloid blast cells, is classified by the World Health Organization as a subtype of acute myeloid leukemia (AML). Isolated, primary, or nonleukemic MS has an incidence of 2/1,000,000 adults and constitutes only 0.7% of all AML cases. MS presentations vary widely, with spinal involvement being rare.
A-year-old male presented with interscapular pain radiating to the right upper arm/neck but was neurologically intact. Once diagnosed with isolated spinal MS, he underwent a surgical decompression followed by local irradiation, systemic chemotherapy, and bone marrow transplantation. Eight months postoperatively, however, he experienced a graft-versus-host rejection and required additional therapies.
Establishing the diagnosis of MS is challenging and typically requires histological confirmation (i.e., the presence of myeloblasts and granulocytic cells). However, optimal treatment strategies remain elusive; despite radiation, chemotherapy, bone marrow transplant/other local therapies, the overall long-term prognosis for MS remains poor.
髓系肉瘤（MS）是一种由髓母细胞组成的罕见髓外肿瘤，世界卫生组织将其归类为急性髓系白血病（AML）的一种亚型。孤立性、原发性或非白血病性MS在成年人中的发病率为2/1000000，仅占所有AML病例的0.7%。MS的表现差异很大，脊柱受累罕见。
一名男性患者因肩胛间疼痛放射至右上臂/颈部就诊，但神经系统检查正常。一旦被诊断为孤立性脊柱MS，他接受了手术减压，随后进行了局部放疗、全身化疗和骨髓移植。然而，术后八个月，他经历了移植物抗宿主排斥反应，需要额外的治疗。
确立MS的诊断具有挑战性，通常需要组织学证实（即存在髓母细胞和粒细胞）。然而，最佳治疗策略仍然难以捉摸；尽管进行了放疗、化疗、骨髓移植/其他局部治疗，MS的总体长期预后仍然很差。
